Hydroxyacyl Coenzyme A Dehydrogenase Beta (HADHb)

MTPB; Mitochondrial Trifunctional Protein,Beta Subunit; 3-Ketoacyl-Coenzyme A Thiolase/Enoyl-Coenzyme A Hydratase(Trifunctional Protein)Beta Subunit; Beta-ketothiolase

SPN	N979
HGNC	4803
MGI	2136381
PubMed	9259266
RGD	620513
UniProt	P55084
Wiki	HADHB

HADHb encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency.

The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Alternatively spliced transcript variants have been found; however, their full-length nature is not known.

Reference

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